-
1
-
-
0024424270
-
Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
-
Riordan, J. R., Rommens, J. M., Kerem, B., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J. L. et al. (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245, 1066-1073
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
2
-
-
0032912589
-
Structure and function of the CFTR chloride channel
-
Sheppard, D. N. and Welsh, M. J. (1999) Structure and function of the CFTR chloride channel. Physiol. Rev. 79, S23-S45
-
(1999)
Physiol. Rev.
, vol.79
-
-
Sheppard, D.N.1
Welsh, M.J.2
-
3
-
-
0032160101
-
Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro
-
Zabner, J., Smith, J. J., Karp, P. H., Widdicombe, J. H. and Welsh, M. J. (1998) Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro. Mol. Cells 2, 397-403
-
(1998)
Mol. Cells
, vol.2
, pp. 397-403
-
-
Zabner, J.1
Smith, J.J.2
Karp, P.H.3
Widdicombe, J.H.4
Welsh, M.J.5
-
4
-
-
0037027912
-
An overview of the pathogenesis of cystic fibrosis lung disease
-
Boucher, R. C. (2002) An overview of the pathogenesis of cystic fibrosis lung disease. Adv. Drug Deliv. Rev. 54, 1359-1371
-
(2002)
Adv. Drug Deliv. Rev.
, vol.54
, pp. 1359-1371
-
-
Boucher, R.C.1
-
5
-
-
0026640380
-
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis
-
Clarke, L. L., Grubb, B. R., Gabriel, S. E., Smithies, O., Koller, B. H. and Boucher, R. C. (1992) Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257, 1125-1128
-
(1992)
Science
, vol.257
, pp. 1125-1128
-
-
Clarke, L.L.1
Grubb, B.R.2
Gabriel, S.E.3
Smithies, O.4
Koller, B.H.5
Boucher, R.C.6
-
6
-
-
0033823118
-
The barium site in a potassium channel by X-ray crystallography
-
Jiang, Y. and MacKinnon, R. (2000) The barium site in a potassium channel by X-ray crystallography. J. Gen. Physiol. 115, 269-272
-
(2000)
J. Gen. Physiol.
, vol.115
, pp. 269-272
-
-
Jiang, Y.1
MacKinnon, R.2
-
7
-
-
0032478818
-
+ conduction and selectivity
-
+ conduction and selectivity. Science 280, 69-77
-
(1998)
Science
, vol.280
, pp. 69-77
-
-
Doyle, D.A.1
Morais Cabral, J.2
Pfuetzner, R.A.3
Kuo, A.4
Gulbis, J.M.5
Cohen, S.L.6
Chait, B.T.7
MacKinnon, R.8
-
8
-
-
0037122805
-
X-ray structure of a CIC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity
-
Dutzler, R., Campbell, E. B., Cadene, M., Chait, B. T. and MacKinnon, R. (2002) X-ray structure of a CIC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity. Nature (London) 415, 287-294
-
(2002)
Nature (London)
, vol.415
, pp. 287-294
-
-
Dutzler, R.1
Campbell, E.B.2
Cadene, M.3
Chait, B.T.4
MacKinnon, R.5
-
9
-
-
0037418859
-
Gating the selectivity filter in CIC chloride channels
-
Dutzler, R., Campbell, E. B. and MacKinnon, R. (2003) Gating the selectivity filter in CIC chloride channels. Science 300, 108-112
-
(2003)
Science
, vol.300
, pp. 108-112
-
-
Dutzler, R.1
Campbell, E.B.2
MacKinnon, R.3
-
10
-
-
0033583055
-
A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules
-
Zerhusen, B., Zhao, J., Xie, J., Davis, P. B. and Ma, J. (1999) A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules. J. Biol. Chem. 274, 7627-7630
-
(1999)
J. Biol. Chem.
, vol.274
, pp. 7627-7630
-
-
Zerhusen, B.1
Zhao, J.2
Xie, J.3
Davis, P.B.4
Ma, J.5
-
11
-
-
0035970113
-
Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction
-
Raghuram, V., Mak, D. D. and Foskett, J. K. (2001) Regulation of cystic fibrosis transmembrane conductance regulator single-channel gating by bivalent PDZ-domain-mediated interaction. Proc. Natl. Acad. Sci. U.S.A. 98, 1300-1305
-
(2001)
Proc. Natl. Acad. Sci. U.S.A.
, vol.98
, pp. 1300-1305
-
-
Raghuram, V.1
Mak, D.D.2
Foskett, J.K.3
-
12
-
-
0036628859
-
CFTR is a monomer: Biochemical and functional evidence
-
Chen, J. H., Chang, X. B., Aleksandrov, A. A. and Riordan, J. R. (2002) CFTR is a monomer: biochemical and functional evidence. J. Membr. Biol. 188, 55-71
-
(2002)
J. Membr. Biol.
, vol.188
, pp. 55-71
-
-
Chen, J.H.1
Chang, X.B.2
Aleksandrov, A.A.3
Riordan, J.R.4
-
13
-
-
0035807019
-
A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator
-
Ramjeesingh, M., Li, C., Kogan, I., Wang, Y, Huan, L. J. and Bear, C. E. (2001) A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator. Biochemistry 40, 10700-10706
-
(2001)
Biochemistry
, vol.40
, pp. 10700-10706
-
-
Ramjeesingh, M.1
Li, C.2
Kogan, I.3
Wang, Y.4
Huan, L.J.5
Bear, C.E.6
-
14
-
-
0026073070
-
Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance
-
Kartner, N., Hanrahan, J. W., Jensen, T. J., Naismith, A. L., Sun, S. Z., Ackerley, C. A., Reyes, E. F., Tsui, L. C., Rommens, J. M., Bear, C. E. et al. (1991) Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance. Cell (Cambridge, Mass.) 64, 681-691
-
(1991)
Cell (Cambridge, Mass.)
, vol.64
, pp. 681-691
-
-
Kartner, N.1
Hanrahan, J.W.2
Jensen, T.J.3
Naismith, A.L.4
Sun, S.Z.5
Ackerley, C.A.6
Reyes, E.F.7
Tsui, L.C.8
Rommens, J.M.9
Bear, C.E.10
-
15
-
-
0035424455
-
Evidence that CFTR channels can regulate the open duration of other CFTR channels: Cooperativity
-
Krouse, M. E. and Wine, J. J. (2001) Evidence that CFTR channels can regulate the open duration of other CFTR channels: cooperativity. J. Membr. Biol. 182, 223-232
-
(2001)
J. Membr. Biol.
, vol.182
, pp. 223-232
-
-
Krouse, M.E.1
Wine, J.J.2
-
16
-
-
0028016412
-
CFTR displays voltage dependence and two gating modes during stimulation
-
Fischer, H. and Machen, T. E. (1994) CFTR displays voltage dependence and two gating modes during stimulation. J. Gen. Physiol. 104, 541-566
-
(1994)
J. Gen. Physiol.
, vol.104
, pp. 541-566
-
-
Fischer, H.1
Machen, T.E.2
-
17
-
-
0028977988
-
- channel is functional when retained in endoplasmic reticulum of mammalian cells
-
- channel is functional when retained in endoplasmic reticulum of mammalian cells. J. Biol. Chem. 270, 12347-12350
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 12347-12350
-
-
Pasyk, E.A.1
Foskett, J.K.2
-
18
-
-
0032530592
-
Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy
-
Eskandari, S., Wright, E. M., Kreman, M., Starace, D. M. and Zampighi, G. A. (1998) Structural analysis of cloned plasma membrane proteins by freeze-fracture electron microscopy. Proc. Natl. Acad. Sci. U.S.A. 95, 11235-11240
-
(1998)
Proc. Natl. Acad. Sci. U.S.A.
, vol.95
, pp. 11235-11240
-
-
Eskandari, S.1
Wright, E.M.2
Kreman, M.3
Starace, D.M.4
Zampighi, G.A.5
-
19
-
-
0028129406
-
Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro
-
Marshall, J., Fang, S., Ostedgaard, L. S. O'Riordan, C. R., Ferrara, D., Amara, J. F., Hoppe, Ht., Scheule, R. K., Welsh, M. J., Smith, A. E. et al. (1994) Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro. J. Biol. Chem. 269, 2987-2995
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 2987-2995
-
-
Marshall, J.1
Fang, S.2
Ostedgaard, L.S.3
O'Riordan, C.R.4
Ferrara, D.5
Amara, J.F.6
Hoppe, Ht.7
Scheule, R.K.8
Welsh, M.J.9
Smith, A.E.10
-
20
-
-
0033567397
-
Novel method for evaluation of the oligomeric structure of membrane proteins
-
Ramjeesingh, M., Huan, L. J., Garami, E. and Bear, C. E. (1999) Novel method for evaluation of the oligomeric structure of membrane proteins. Biochem. J. 342, 119-123
-
(1999)
Biochem. J.
, vol.342
, pp. 119-123
-
-
Ramjeesingh, M.1
Huan, L.J.2
Garami, E.3
Bear, C.E.4
-
21
-
-
0034724192
-
Detection of β2-adrenergic receptor dimerization in living cells using bioluminescence resonance energy transfer (BRET)
-
Angers, S., Salahpour, A., Joly, E., Hilairet, S., Chelsky, D., Dennis, M. and Bouvier, M. (2000) Detection of β2-adrenergic receptor dimerization in living cells using bioluminescence resonance energy transfer (BRET). Proc. Natl. Acad. Sci. U.S.A 97, 3684-3689
-
(2000)
Proc. Natl. Acad. Sci. U.S.A.
, vol.97
, pp. 3684-3689
-
-
Angers, S.1
Salahpour, A.2
Joly, E.3
Hilairet, S.4
Chelsky, D.5
Dennis, M.6
Bouvier, M.7
-
22
-
-
0031031039
-
Surface expression of the AMPA receptor subunits GluR1, GluR2, and GluR4 in stably transfected baby hamster kidney cells
-
Hall, R. A., Hansen, A., Andersen, P. H. and Soderling, T. R. (1997) Surface expression of the AMPA receptor subunits GluR1, GluR2, and GluR4 in stably transfected baby hamster kidney cells. J. Neurochem. 68, 626-630
-
(1997)
J. Neurochem.
, vol.68
, pp. 626-630
-
-
Hall, R.A.1
Hansen, A.2
Andersen, P.H.3
Soderling, T.R.4
-
23
-
-
0034730330
-
Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity
-
Wang, S., Yue, H., Derin, R. B., Guggino, W. B. and Li, M. (2000) Accessory protein facilitated CFTR-CFTR interaction, a molecular mechanism to potentiate the chloride channel activity. Cell (Cambridge, Mass.) 103, 169-179
-
(2000)
Cell (Cambridge, Mass.)
, vol.103
, pp. 169-179
-
-
Wang, S.1
Yue, H.2
Derin, R.B.3
Guggino, W.B.4
Li, M.5
-
24
-
-
0037422547
-
A macromolecular complex of β2-adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA
-
Naren, A. P., Cobb, B., Li, C., Roy, K., Nelson, D., Heda, G. D. Liao, J., Kirk, K. L., Sorscher, E. J., Hanrahan, J. et al. (2003) A macromolecular complex of β2-adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA. Proc. Natl. Acad. Sci. U.S.A. 100, 342-346
-
(2003)
Proc. Natl. Acad. Sci. U.S.A.
, vol.100
, pp. 342-346
-
-
Naren, A.P.1
Cobb, B.2
Li, C.3
Roy, K.4
Nelson, D.5
Heda, G.D.6
Liao, J.7
Kirk, K.L.8
Sorscher, E.J.9
Hanrahan, J.10
-
25
-
-
1842333889
-
Genotype and phenotype in cystic fibrosis
-
Tsui, L. C. and Durie, P. (1997) Genotype and phenotype in cystic fibrosis. Hosp. Pract. (Off. Ed.) 32, 115-118, 123-129, 134
-
(1997)
Hosp. Pract. (Off. Ed.)
, vol.32
, pp. 115-118
-
-
Tsui, L.C.1
Durie, P.2
-
26
-
-
0031915434
-
Limited proteolysis as a probe for arrested conformational maturation of δ F508 CFTR
-
Zhang, F., Kartner, N. and Lukacs, G. L. (1998) Limited proteolysis as a probe for arrested conformational maturation of δ F508 CFTR. Nat. Struct. Biol. 5, 180-183
-
(1998)
Nat. Struct. Biol.
, vol.5
, pp. 180-183
-
-
Zhang, F.1
Kartner, N.2
Lukacs, G.L.3
-
27
-
-
0026907529
-
Mislocalization of δ F508 CFTR in cystic fibrosis sweat gland
-
Kartner, N., Augustinas, O., Jensen, T. J., Naismith, A. L. and Riordan, J. R. (1992) Mislocalization of δ F508 CFTR in cystic fibrosis sweat gland. Nat. Genet. 1, 321-327
-
(1992)
Nat. Genet.
, vol.1
, pp. 321-327
-
-
Kartner, N.1
Augustinas, O.2
Jensen, T.J.3
Naismith, A.L.4
Riordan, J.R.5
-
28
-
-
0026781952
-
Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
-
Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E. and Welsh, M. J. (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature (London) 358, 761-764
-
(1992)
Nature (London)
, vol.358
, pp. 761-764
-
-
Denning, G.M.1
Anderson, M.P.2
Amara, J.F.3
Marshall, J.4
Smith, A.E.5
Welsh, M.J.6
-
29
-
-
0025242929
-
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
-
Cheng, S. H., Gregory, R. J., Marshal, J., Paul, S., Souza, D. W., White, G. A., O'Riordan, C. R. and Smith, A. E. (1990) Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell (Cambridge, Mass.) 63, 827-834
-
(1990)
Cell (Cambridge, Mass.)
, vol.63
, pp. 827-834
-
-
Cheng, S.H.1
Gregory, R.J.2
Marshal, J.3
Paul, S.4
Souza, D.W.5
White, G.A.6
O'Riordan, C.R.7
Smith, A.E.8
|